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A new case of pseudo-diastrophic dysplasia in a boy born to consanguineous parents is reported. The patient developed neurologic (hydrocephalus) and respiratory anomalies and died at the age of 11 months of an unknown cause. Features that distinguish this chondrodysplasia from diastrophic dysplasia include decreased height of vertebral bodies and anomalies of the pelvis with short iliac wings and a horizontal orientation of the internal spine of the acetabular roof.  相似文献   
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During biological monitoring of exposure to a chemical, a possible source of interindividual variability in the measurement of a urinary metabolite that undergoes enterohepatic cycling is the presence of dietary fiber in the gastrointestinal tract. This study examined the effect of diets containing either the insoluble fiber Alphacel (nonnutritive bulk cellulose) or the soluble pectin (from citrus fruit, MW 20,000-40,000). Five groups of male Sprague-Dawley rats received one of the following diets: poor (5% w/w) or rich (15% w/w) in Alphacel, poor (5% w/w) or rich (15% w/w) in pectin, or no fiber (NF). Five micromol/kg of pyrene was administered by iv injection immediately after feeding the animals with their respective diet, and urine and feces collections started for the determination of 1-hydroxypyrene (1-OHP), a metabolite of pyrene. The type of fiber had no influence on the results. The rats receiving diets both poor and rich in fiber excreted less 1-OHP (18 +/- 8 and 15 +/- 7 pmol per g of rat, respectively) in the 24-h urine samples than the NF group (28 +/- 6 pmol/g). There was a nonstatistically significant trend towards increased fecal and total (urinary + fecal) 1-OHP excretion with increasing amount of fiber in the diet. An in vitro experiment showed an inverse correlation (r(2) = 0.98) between the amount of Alphacel in suspension in a 1-OHP aqueous solution and the recovery of 1-OHP from the soluble fraction. The reduction in urinary output of the metabolite due to fiber reaching approximately 40% may contribute to its interindividual variability observed in occupational and environmental studies.  相似文献   
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During locomotion, a top-down organization has been previously demonstrated with the head as a stabilized platform and gaze anticipating the horizontal direction of the trajectory. However, the quantitative assessment of the anticipatory sequence from gaze to trajectory and body segments has not been documented. The present paper provides a detailed investigation into the spatial and temporal anticipatory relationships among the direction of gaze and body segments during locomotion. Participants had to walk along several mentally simulated complex trajectories, without any visual cues indicating the trajectory to follow. The trajectory shapes were presented to the participants on a sheet of paper. Our study includes an analysis of the relationships between horizontal gaze anticipatory behavior direction and the upcoming changes in the trajectory. Our findings confirm the following: 1) The hierarchical ordered organization of gaze and body segment orientations during complex trajectories and free locomotion. Gaze direction anticipates the head orientation, and head orientation anticipates reorientation of the other body segments. 2) The influence of the curvature of the trajectory and constraints of the tasks on the temporal and spatial relationships between gaze and the body segments: Increased curvature resulted in increased time and spatial anticipation. 3) A different sequence of gaze movements at inflection points where gaze plans a much later segment of the trajectory.  相似文献   
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The clinical observation describes the case of a 20-year-old woman who has consulted for aesthetic and functional reasons. She presents a skeletal class III normodivergent, an occlusal class III with a lower proalveoli quite marked. In addition, a lingual dysfunction which manifests itself by important anterior diastema and dento-dental disharmony at the upper jaw complicates the case. The undertaken therapeutic project starts with a first step of a lingual praxis rehabilitation, followed by an orthodontic step and upper lateral incisors-plasty. The purpose of those results is the evaluation of the stability two years later, which was reported positive.  相似文献   
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